Cleidocranial dysplasia – the Jerusalem Approach: part 2

Published: October 2013

Bulletin
#26 October 2013

Cleidocranial dysplasia – the Jerusalem
Approach: part 2

Part 1 of this short series of articles on the treatment of
Cleidocranial dysplasia can be found in the September 2013 bulletin #25 on this
website. It was pointed out there that, for a good proportion of Cleidocranial
dysplasia patients, the jaws are normally related to one another in the
sagittal plane. Nevertheless, the prevalence of a skeletal class 3 discrepancy
is unusually high in these patients and its early orthopedic treatment was discussed
in part 1.

It was emphasized that treatment aimed at erupting the
multiple impacted teeth should be delayed until the patient’s dental age
reaches 7 years. This is the age in the normal child at which the permanent
incisors erupt and it coincides with development of approximately ½ – ¾ the
final length of the completed root.1-3 Since tooth development in
CCD is usually about 3 years delayed 4-6 in relation to the
unaffected child, this means that treatment for the resolution of the multiple
impactions is not advised until the CCD patient reaches 10 years of age. Thus a
window of opportunity presents to initiate maxillary protraction in the very
young patient, aimed at treating the developing skeletal class 3 problem which,
in CCD, is mainly due to maxillary hypoplasia.

In some, the attempt to treat the Class III dysgnathic
relation may have failed due to poor cooperation of the patient with wearing
the face mask. In these circumstances it is frequently more advantageous, in
the long run, to foreclose on the protraction attempt. It is probably more beneficial
to renew the momentum of treatment on another facet of CCD and to try to
achieve some recognizable progress by redirecting to the eruption of teeth, in order
to revamp the patient’s motivation for treatment, rather than to fight the
uphill orthopedic battle with poor compliance and no improvement in sight. In
others, the patient may arrive at a dentist’s doorstep to seek treatment for
the first time only at around the age of 10 years, when erupting the individual
teeth will need to take precedence over attempting skeletal orthopedic correction.

Fig. 1.Intra-oral
views of the dentition of a 13 year old CCD male patient, showing a complete
deciduous dentition and only the erupted first molars of the permanent
dentition. The clinical condition indicates a mild skeletal Class III inter-arch
relation.

CCD patients attend their first consultation with a variety
of over-retained deciduous teeth in the mouth. By and large, the first
permanent molars erupt in all four quadrants4, 5 and, in addition,
there may be one or more of the permanent incisors in a state of partial
eruption, although an intact and complete deciduous dentition is not unusual (Fig.
1).With an older patient, there may be
more erupted or partially erupted incisors and the posterior deciduous teeth
are frequently affected by caries. A panoramic radiograph will reveal the
presence of all the unerupted permanent teeth, while a careful examination will
usually permit the identification of several supernumerary teeth, particularly
in the maxillary midline area, but often elsewhere, too (Fig. 2). In some
cases, the crowding that is produced by the multitude of unerupted teeth causes
the tooth germs of many of them to be displaced deep into basal bone, including
those of the normal series. Without intervention, the close proximity of the
developing roots to the lower border of the mandible or to the floor of the
nose may result in stunted or distorted root configurations.

In line with normal dental development in an unaffected
child, it is desirable that the teeth in the CCD patient erupt into the mouth
at the time when there is an open apex and one-half to three-quarters of the
eventual expected root length. At the age of 10 years, the only teeth answering
to these criteria are the incisors in both jaws. The premolar teeth are not
expected to reach this level of development for a further 3 or 4 years. Thus
any attempt to surgically expose and orthodontically erupt all incisors,
canines and premolars in a single surgical episode, would be strongly
contraindicated. On the other hand, to delay the treatment for 3 or 4 years, to
the same end, would mean leaving the incisors and their associated
supernumerary teeth to develop their roots in extremely cramped circumstances.
With their root apices becoming fully closed, they would lose any of the
already reduced eruptive potential that they may have initially possessed.

A more serious result of complete elimination of all the deciduous
teeth and all the buried supernumerary teeth is the fact that the patient would
be almost totally edentulous for a lengthy period of time, with only the
permanent molars available to support an orthodontic appliance. This would
severely tax the resources available to devise a biomechanical system needed to
vertically erupt the permanent teeth - not to mention the debilitating, functional
and social aspects of such a drastic line of treatment.

In the Jerusalem method of treatment4, 5, 7, surgery
is performed in two distinct stages. The timing of these surgical episodes is
directly related to the dental age at which the different groups of teeth reach
the point in time at which they should be normally erupting, i.e. ½ – ¾ of their
eventual root development. Thus, the first surgical episode is planned for
dental age 7 years, which translates into the patient’s chronologic age of 10
years, in most cases and involves the incisor teeth of both jaws only.

In this way, the first permanent molars and the deciduous
canines and molars remain in place to provide the patient with the means to
function and also to provide the orthodontist with several available teeth to
use as support and anchorage for the orthodontic appliances.Mechanically efficient application of vertically
directed force may then be brought to bear on the unerupted incisors.

The second surgical episode will be planned when the canines
and premolars realize the same root development requirement, i.e. 3-4 years
later. By that time, the incisor teeth will have been fully erupted and
aligned, to provide the patient with adequate function and appearance, but also
to permit the inclusion of these teeth into the support and anchor unit of the
orthodontic appliance for the efficient eruption and alignment of the posterior
teeth.

This month’s bulletin deals with the incisor phase of tooth
eruption, the orthodontic appliances needed for its effective execution, the
requirements of the surgical procedure and the tasks that must be fulfilled
before the patient is wheeled out of the operating theatre. It also proceeds to
discuss ways in which to successfully erupt the teeth and then to align them in
the dental arch and into a suitable archform.

Preparing the patient

Fig. 2a. poor quality cephalogram at 12 years of age shows a steep
mandibular plane and vertical growth direction. The mass of superimposed,
unerupted teeth can be clearly seen to be at considerable distances from the
occlusal plane. The top part of the film has been deliberately
“photoshopped”to illustrate the open
anterior fontanelle and coronal suture.

Fig. 2b The initial panoramic view

Fig. 2c. A tracing of the panoramic view in Fig. 2b with the deciduous
teeth labeled A-E, the permanent teeth labeled 1-8 and the supernumerary teeth
asterisked.

For the purposes of illustrating the method, we will describe a dentition
exhibiting the “worst case scenario”, meaning that, aside from erupted first
permanent molars, the only other erupted teeth are the deciduous teeth
incisors, canines and molars – 20 in all (Fig. 1). An initial examination with
the pediatric dentist needs to be arranged at which the permanent molars,
deciduous molars and deciduous canines are examined for caries and treated
restoratively in the normal way, while oral hygiene is reinforced and any other
necessary preventive measures are instituted. It should be remembered that these
deciduous teeth will need to be maintained in a healthy state for a further 3-4
years. The orthodontist will require a panoramic view and a cephalometric
radiograph, but it is more than likely that these will be available from visits
to other medical and dental practitioners (Fig. 2a, b). It should be borne in
mind that the CCD patient will be required to have many radiographs and CBCT’s
both for diagnosis and for follow-up of the treatment during the years ahead.
Monitoring of the number and type of films that are needed should be carried
out and care exercised in commissioning new films in order to reduce the considerable
dosage of ionizing radiation to which the patient will inevitably be subjected.

The initial films need to be carefully studied to identify
the teeth of the normal series and the supernumerary teeth. The individual
teeth on the film should be traced, labeled accordingly and the case discussed
with the surgeon in regard to the upcoming first surgical phase (Fig. 2c). A
decision needs to be made regarding which teeth will be extracted, which
surgically exposed and which exposed-and-attachment-bonded. A cone beam CT will
often be an essential diagnostic aid at this stage and, for the most part
should be considered mandatory.

Appliance construction

It is essential that the patient should go into the
operating theatre set up with the adequate initial construction in place, from
which to apply extrusive forces to the impacted teeth. This is best initiated
during the actual surgical procedure itself and before the patient is aroused
from the anesthetic. If this is attempted later, there is a very strong chance
of failure due to the fact that the patient is fully conscious, soft tissues
tend to close over any partially erupted teeth and there is considerable
tenderness of the recently surgically traumatized oral soft tissues – even
several weeks later.

Fig. 3. The basic
appliance

a.Maxillary and
mandibular appliances consist of palatal and lingual arches soldered to bands
fitted on the first permanent molars. Round buccal tubes of internal diameter
0.036” (0.9mm) are soldered to the buccal sides of the bands. Removable, heavy
(0.036”), self-supporting archwires are fashioned with a bayonet bend stop at
the molar tubes.

b, c detail of the individual archwires, showing
the bayonet bends on each side, a mesially and distally facing hook in each of
the canine areas and an anterior soldered wire frame.

Separating elastics are inserted interproximally in the
molar areas and, a few days later, plain bands are adapted to the four molar
teeth. An alginate or compound impression is made of each arch with the bands
in place and the bands are then removed and relocated in the impression before
the model is poured. On the cast models, a lower lingual and upper palatal arch
of 0.036” heavy steel wire are constructed and soldered to the lingual side of
the bands. Round 0.036” tubes are then soldered to the buccal side of the bands
in each jaw. A heavy labial archwire is then fashioned in 0.036” round
cross-section steel wire and slotted into the buccal tubes on each side. The
wire is prevented from sliding too far into the tube by a U-loop on each side of the archwire, which is
designed to hold the anterior portion of the heavy archwire 2 or 3 millimeters
labial to the deciduous incisors. A distally-facing hook is soldered in the
canine area on each side of the archwire and a small wire frame is soldered in
the midline area. This represents the basic appliance (Fig. 3) and, when placed
in the mouth, is entirely passive. The molar bands and lingual arches should be
cemented into place a week or so before the proposed surgery, to be sure that
all is well. The removable labial archwires are not inserted at this stage but will
be taken to the operating theatre separately.

A good alternative to this system requires the placement of
orthodontic brackets on the deciduous molars and canines at a uniform height,
so that a plain archwire may be introduced immediately. No brackets are placed
on the deciduous incisors.

Surgery and interdisciplinary cooperation

As noted above, many impacted teeth in CCD patients are to
be found very deep into the basal bone. To follow an open exposure policy would
entail removing the supernumerary teeth and all the bone around the impacted
teeth of the normal series down to the CEJ. It also means maintaining the
patency of the exposure by reducing the soft tissue and placing surgical packs,
together with the need to suture the surgical flaps to prevent the gingival
tissues from re-covering the exposed teeth. To do this for a series of deeply
impacted teeth would mean paring the height of the alveolar bone down to the
level of the necks of these teeth. This would result in a very radical removal
of bone and a potentially dangerous weakening of the body of the mandible, in
particular

Fig. 4. Surgery was performed in both jaws
together, in July 2006, by Prof. J. Lustmann and Dr. E Regev. Surgery in the
mandible:

a.A full labial flap
is reflected from the gingival margins of the deciduous teeth

b.The crowns of the
four incisors are exposed. Because supernumerary teeth were extracted, the
degree of crown exposure on one or two of the normal teeth was relatively
large. In this case, the patient’s age
was more advanced and the canines more developed, corresponding with 2-3 years
beyond the ideal time. Accordingly, the canines were also exposed in this first
surgical stage. Note the extreme height difference between the deciduous and
the permanent anterior teeth.

c.Eyelet attachments
were bonded by the orthodontist and oriented in line with the long axis of the
teeth.

d.The removable labial
arch is re-inserted and lies in its passive state.

e.With light finger
pressure, the anterior portion of the heavy labial arch is gently flexed
inferiorly and the twisted ligature wires engage it by bending them over the
archwire, thereby holding it down under light force. An extrusive force is now
being applied to the impacted anterior teeth. The soldered lingual arch can be
clearly seen, superior to the activated labial arch.

A closed surgical exposure policy is strongly recommended
for these teeth (Fig.4, 5). This means opening an attached gingiva flap from
the gingival crevice of the deciduous teeth, following their extraction,
identifying the crypts of the teeth deep in the sulcus and removing only the thin
shell of bone on the buccal aspect of the teeth. The follicle is opened only on
this aspect and only enough to provide enamel surface large enough to accept a
small bonded attachment. Bone is not removed occlusally nor interproximally and
bone channeling in the intended direction of tooth movement is avoided. Every
effort is made to conserve as much bone as possible, in the interests of
maintaining and, eventually, enhancing alveolar bone height.

Fig.
5. Surgery in the maxilla:

a-f.Parallel stages as per the mandibular
surgery.

g.The extracted
deciduous and supernumerary teeth from the surgery.

h.The immediate
post-surgery panoramic view

Long term experience has shown and it is emphasized that overlying
bone is not an impediment to the movement of the impacted teeth. It resorbs in
response to the vertical extrusive forces in the same way as forces acting on
erupted teeth in routine orthodontic practice.8, 9

The orthodontist should be on hand to bond simple eyelets to
the exposed and bonded incisor teeth, with the eyelets oriented parallel to the
long axes of the teeth concerned (Fig.4c, 5c,d). The pigtail ligatures that are
twisted into the eyelets are drawn vertically, while the removable archwires
are reinserted into the horizontal buccal tubes (Fig. 4d, 5e). The pigtail
ligatures are then bent over to ensnare the heavy labial archwire under some pressure,
which deflects the archwire towards the impacted teeth (Fig. 4e, 5f). The
archwire is now loaded with an extrusive force. The surgeon then closes and
sutures back the full flap to cover the entire exposed surgical field.

Fig. 5g. The extracted deciduous and supernumerary teeth.

Fig. 5h. The immediate post-surgery panoramic view

With the alternative method, in which brackets had been
placed on the deciduous molars and canines, a fairly substantial (0.016” or
0.018” stainless steel) archwire is ligated into to all the brackets, with the
twisted ligatures hooked over the free anterior portion.

Back in the Orthodontic Clinic

The patient will be seen by the surgeon in a post-surgical
visit, prior to releasing him/her home. A further visit will generally be
scheduled for follow-up of healing and removal of sutures a week or so later
and then the patient will be referred back to the orthodontist for the
continuation of the treatment.

The anchorage employed in the initial activation of the
heavy archwire is from the molar teeth and from the lingual and palatal arches.
If this is not reinforced with effective supplemental resistance, the molars
will tip mesially and, in the maxilla in particular, the palatal arch will
impinge on the palatal tissue and may sometimes become buried in it. The
alternative method of placing orthodontic brackets on the deciduous molars and
canines will reduce, but not eliminate, the likelihood of this complication.

On the first post-surgical visit to the orthodontist,
usually 3-4 weeks later, the tissues will be tender and the patient very
apprehensive. For these reasons, it is wise to limit the treatment provided at
this appointment to cleaning the general surgical area with atomized water from
the triple syringe. If one or more of the pigtail ligatures are causing
ulceration of the lips or tongue, they should be carefully rolled up with a
plier or ligature director. However, it is often wise to use surface anesthetic
spray to limit the discomfort engendered by the manipulation.

Fig. 6. The upper and lower anterior teeth seen here to be erupting, in
February 2007. Note the use of a
single anterior up-and-down elastic, placed on the distal-facing hooks on each
side of both jaws and being held away from the tissues in the midline by the soldered
wire frame. Intermaxillary vertical elastics represent the most important and
most efficacious means of applying vertical extrusive force to the anterior
teeth in both jaws.

The best way to guarantee the effectiveness of the anchor
unit is to apply vertical “up-and-down” elastics to the labial arches, whether
heavy archwires or the partially bracketed appliance system (Fig. 6). Aside
from anchorage considerations, intermaxillary vertical elastics also have a
very positive effect on the extrusive forces that have been applied to the
teeth. For this reason, the first post-surgical visit is also the first real
opportunity to teach the child to place up-and-down elastics.The distal-facing hooks on the heavy
archwires were designed with this in mind. A single 3/8” or 5/16” medium
elastic is stretched from the right to left hooks in the maxilla and then
vertically down to engage the parallel hooks on the mandibular archwire. To
keep the middle part of the elastic clear of the healing gingiva, it is
stretched labially over the small soldered wire frame in the midline.

In the bracketed system, right and left small up-and-down
elastics (3/16” heavy) must be placed on prepared hooks on the deciduous canine
and first deciduous molar brackets.

Further activation should be made every 3-4 weeks, in line
with the progress towards the eruption of the teeth. As the pigtail ligatures become
longer and as the initial activation becomes exhausted, they require to be
rolled up to maintain the momentum of the extrusive force. The teeth will come
through fairly quickly, depending upon their initial degree of displacement and
ectopy, the range and magnitude of the force and the frequency with which
adjustments are made. They erupt in a markedly lingual position and with a
lingual inclination, because the point of application of the extrusive force is
to a labial attachment.

The vertically-oriented eyelets, that had been bonded to the
teeth at the surgical procedure, become fully visible supra-gingivally when the
incisor teeth are fairly well erupted. At this point the heavy archwire should
be discarded and regular brackets placed on the deciduous molars and canines at
a uniform height, as with the alternative bracketed system. The two systems are
now identical.

Fig. 7.In
November 2007 and once the eyelet attachments had become fully exposed
supragingivally, standard Tip-Edge orthodontic brackets (TP Orthodontics) were
placed and routine leveling and alignment commenced, using a two-by-four
Johnson-type appliance. The new permanent teeth typically erupt with a strong
lingual inclination and expanded coil springs were placed on the buccal arms to
procline them and to achieve good archform. This also provides space for
eruption of the canines (arrows).At
the same time, vertical up-and-down elastics were re-instituted to close down
the wide open bite.

A flexible superelastic nickel-titanium wire of 0.012” or
0.014” gauge should now be threaded through each of the eyelets of all four
erupted incisors and tied into the remaining brackets in the normal way. This
step requires just one visit for it to be immensely effective at leveling the
teeth and eliminating even gross rotations where there is no room for the
placement of a bracket in an ideal position. At the next visit, the eyelets
should be debonded and regular orthodontic brackets substituted (Fig. 7).Simple labial tipping of the anterior teeth
in both arches will correct their post-eruptive lingual inclination. It will
have the effect of establishing, for the first time, a normal archform and a
normal incisor height. Space in the posterior region will be increased to
provide place for the eventual eruption of the permanent canines and premolars.

Fig. 8. Panoramic
view of August 2008 of August 2008 shows the premolar teeth with largely
completed root apices and, therefore, late for the second orthodontic/surgical
phase. Note also that the right mandibular second molar has fully erupted and
that the other second molars appear to have a good prognosis for spontaneous
eruption, which is frequent in CCD patients. Note also the development of a 4th
maxillary molar on each side, apart from the 3 supernumerary teeth in the
premolar areas.

The treatment time for this first stage of treatment ranges
between 1.5 – 2.5 years, depending on the severity of the condition at the
outset and the efficiency of the mechanotherapy. The patient may now be ready,
in terms of the maturation of the premolar and canine teeth, to continue on to
the second stage of treatment. However, for the most part, there will be a
further 2 years or more before the treatment may be renewed.

In many cases, alignment, leveling and uprighting of the
teeth will have been achieved while realizing the above goals. However, once
these main goals have been attained, all treatment should stop and any
uncompleted root movements, particularly bucco-lingual root torque, should be
relegated to the second stage of treatment, when all necessary root movements
will be tackled simultaneously for all the erupted teeth (Figs. 8, 9).

Fig. 9.Completion
of the first orthodontic/surgical phase of treatment, with good alignment and
archform and adequate bite closing achieved. Note the worsening skeletal Class III relation.

If the oral hygiene has been good, the caries rate low and
the estimated time span between stages will not be too long, the appliances may
be left in place and remain as retainers. Otherwise, they should be removed and
simple removable retainers placed until the second stage may begin.